Please use this identifier to cite or link to this item:
Title: CoQ deficiency causes disruption of mitochondrial sulfide oxidation, a new pathomechanism associated with this syndrome.
Authors: Luna-Sánchez, Marta
Hidalgo-Gutiérrez, Agustín
Hildebrandt, Tatjana M
Chaves-Serrano, Julio
Barriocanal-Casado, Eliana
Santos-Fandila, Ángela
Romero, Miguel
Sayed, Ramy Ka
Duarte, Juan
Prokisch, Holger
Schuelke, Markus
Distelmaier, Felix
Escames, Germaine
Acuña-Castroviejo, Darío
López, Luis C
Keywords: COX;SQR;blood pressure;glutathione;mitochondrial disease
metadata.dc.subject.mesh: Animals
Blood Pressure
Cells, Cultured
Disease Models, Animal
Mitochondrial Diseases
Muscle Weakness
Quinone Reductases
Issue Date: 2017
Abstract: Coenzyme Q (CoQ) is a key component of the mitochondrial respiratory chain, but it also has several other functions in the cellular metabolism. One of them is to function as an electron carrier in the reaction catalyzed by sulfide:quinone oxidoreductase (SQR), which catalyzes the first reaction in the hydrogen sulfide oxidation pathway. Therefore, SQR may be affected by CoQ deficiency. Using human skin fibroblasts and two mouse models with primary CoQ deficiency, we demonstrate that severe CoQ deficiency causes a reduction in SQR levels and activity, which leads to an alteration of mitochondrial sulfide metabolism. In cerebrum of Coq9R239X mice, the deficit in SQR induces an increase in thiosulfate sulfurtransferase and sulfite oxidase, as well as modifications in the levels of thiols. As a result, biosynthetic pathways of glutamate, serotonin, and catecholamines were altered in the cerebrum, and the blood pressure was reduced. Therefore, this study reveals the reduction in SQR activity as one of the pathomechanisms associated with CoQ deficiency syndrome.
metadata.dc.identifier.doi: 10.15252/emmm.201606345
Appears in Collections:Producción 2020

Files in This Item:
File SizeFormat 
PMC5210161.pdf3,66 MBAdobe PDFView/Open

This item is protected by original copyright

This item is licensed under a Creative Commons License Creative Commons